Persistent Autobiographical Amnesia: a Case Report

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Behavioural Neurology 18 (2007) 13–17 13 IOS Press

Persistent autobiographical amnesia: A case report

C. Repettoa,∗, R. Manentib, V. Sansonec, M. Cotellia,d, D. Peranie, V. Garibottoe, O. Zanettia, G. Meolac and C. Miniussia,f aIRCCS San Giovanni di Dio FBF, Brescia, Italy bVita Salute University and San Raffaele Scientiﬁc Institute, Milan, Italy cDepartment of Neurology, Policlinico San Donato, University of Milan, Italy dCognitive Science, University of Turin, Italy eIRCCS San Raffaele Hospital, Milan, Italy f Department of Biological Sciences and Biotechnologies, University of Brescia, Italy

Abstract. We describe a 47-year-old man who referred to the Emergency Department for sudden global amnesia and left mild motor impairment in the setting of increased arterial blood pressure. The acute episode resolved within 24 hours. Despite general recovery and the apparent transitory nature of the event, a persistent selective impairment in recollecting events from some specific topics of his personal life became apparent. Complete neuropsychological tests one week after the acute onset and 2 months later demonstrated a clear retrograde memory deficit contrasting with the preservation of anterograde memory and learning abilities. One year later, the autobiographic memory deficit was unmodified, except for what had been re-learnt. Brain MRI was normal while H20 brain PET scans demonstrated hypometabolism in the right globus pallidus and putamen after 2 weeks from onset, which was no longer present one year later. The absence of a clear pathomechanism underlying focal amnesia lead us to consider this case as an example of functional retrograde amnesia.

Keywords: Functional retrograde amnesia, FRA, memory, autobiographic amnesia

1. Introduction Amnesia (TGA) that from a neuropsychological point of view could share some features with other forms of Organic amnesia is a syndrome in which the patient amnesia, but clinically is a well distinct picture. shows normal intelligence and cognitive profile associ- In some cases amnesia occurs in association with ated to different degrees of impairment in anterograde a psychological aetiology: this non-neurological syn- and retrograde memory. drome is variously named as “hysterical amnesia”, Further relevant information comes from single case “psychogenic amnesia”, “dissociative amnesia” and, studies of patients with memory deficits. In this cate- more recently, “functional amnesia” [16,17,22]. gory we can include acquired disorders, brought about by herpes encephalitis, severe hypoxia, certain vascular We describe a 47-year-old man, who referred to the lesions, deep midline tumors, head injury, basal fore- Emergency Department for sudden global amnesia and brain lesions or degenerative brain disease. A partic- left mild motor impairment in the setting of increased ular category of organic amnesia is Transient Global arterial blood pressure. Complete neuropsychological tests one week after the acute onset and 2 months later demonstrated a clear retrograde memory deficit con- ∗ Corresponding author: Claudia Repetto, IRCCS Fatebenefratelli trasting with the preservation of anterograde memory S. Giovanni di Dio, Via Pilastroni, 4 Brescia, Italy. Tel.: +39 0303501594; Fax: +39 0303501513; E-mail: claudia.repetto@ and of learning abilities, which was unchanged one year cognitiveneuroscience.it. later, except for what had been re-learnt.

2. Case history gressive Matrices, CPM), auditory language comprehension (Token Test), verbal fluency with phonemic 2.1. Initial assessment. and semantic cues, verbal and spatial short-term memory (Digit Span forward and Spatial Span), spatial long A.M. is a right handed, 47-yars-old male who works term memory (Rey Recall), verbal learning (Serial Po- as business consultant (17 years of educational train- sition Curve) and constructional abilities (Rey’s com- ing) and lives with his family (wife and one daughter) plex copy) [19]. in the south of Italy. His developmental history is un- MMSE was normal. The patient showed a good remarkable. Family history is positive for high arterial performance on long term verbal and spatial memory, blood pressure and diabetes. Past medical history in- short term verbal memory and language production. A cludes increased arterial blood pressure six years prior normal score was also obtained at the Serial Position admission to our Clinic but this has not been treated Curve, following the Capitani et al. standardization [5]. and the patient remained asymptomatic. There is no Language comprehension and abstract visuospatial rea- past history of psychiatric abnormalities, significant soning abilities were at a borderline level. Instead, his psycho-pathological traits or alcohol abuse. scores at the tests that assess short term spatial memory The day of referral to the Emergency Department, and constructional apraxia were under the score of the he woke up as usual, had his breakfast and took his normal reference population. daughter to school. The last call on his mobile phone is Although general evaluation showed a global cogni- reported at 9.30 a.m. From 9.38 a.m. his phone became tive functioning preserved, with only isolated deficits unreachable. Instead of meeting with a client as sched- with regard to short term spatial memory and con- uled, he travelled for over 1000 Km to Milan, where he structional abilities, the patient appeared very confused arrived after 12 hours. At that point he asked for help about his personal history: he didn’t remember who he to the police, saying that he was confused and he didn’t was, where he lived and which was his job. For this remember where he lived. reason, it has been considered interesting to analyze He was admitted to the Emergency Department at thoroughly remote memory. our Hospital site. When his wife, advised by police about the finding of her husband, reached him at the 2.3. Neuropsychological evaluation: memory hospital, he didn’t recognize her. Neurological examination at that time revealed mild motor impairment on In order to investigate semantic memory, the the left arm. He was restless and confused. Global WAIS [26] information subtest was also administered: amnesia for autobiographic memories became evident. the performance was comparable to the one of the ref- He was unable to give his name, address and any infor- erence population. Of note, in the Famous Face Recog- mation concerning his family and professional activity. nition Test [19] the patient’s score was normal: he was He failed to recognize any of his relatives when they ar- able to indicate, among four alternatives, the face corre- rived and he hadn’t a sense of familiarity for them. His sponding to the famous name pronounced by the exam- blood pressure on admission was 170/120 and failed iner. The patient’s autobiographical memory was tested to decrease despite treatment for 2 hours. Brain CT on a formal questionnaire, the Autobiographical Mem- scan was normal just like echodoppler of carotid and ory Inventory [15]. The AMI investigates personal vertebral arteries. EEG recorded bilateral and predom- semantic information and autobiographic episodes re- inantly frontal slow waves. Brain MRI was normal. called from three periods of life: childhood, early adult Lumbar puncture was normal. The patient underwent life and recent life. The same questionnaire was ad- a complete neuropsychological evaluation. ministered to his wife, in order to check the veracity of the patient’s recollections. 2.2. Neuropsychological evaluation: general The patient couldn’t remember specific information of his personal history, i.e. which was his job and the Neuropsychological testing was administered by an name of clients and colleagues, suggesting a seman- experienced examiner in a quiet environment in the hos- tic memory deficit. In particular, he said to have a pital. Approximately 60 to 90 minutes were needed to son, instead of a daughter. He couldn’t explain which administer the tests. It included a screening test for de- were the activities of his job and when he was asked mentia (Mini-Mental State Examination, MMSE [9]), about his degree and the topics he had studied and ap- tests of nonverbal reasoning (Raven’s Coloured Pro- plied every day in his working activities , nothing but C. Repetto et al. / Persistent autobiographical amnesia: A case report 15 a sensation of distress was reported. Furthermore, he in order to recall it. Neuropsychological tests were showed a corresponding deficit on episodic memory: repeated. with respect to the same topics (family, job and degree), The AMI interview underlined the same mnestic he had no available memories of detailed events. The gaps as observed few months before. The anterograde most intriguing aspect of his assessments was the lack verbal and non verbal, long and short term, memory of emotional participation on topics regarding his fam- was preserved, even if the patient showed a qualitative ily: there was no apparent pleasure in spending time different behaviour during the assessment: he seemed with his daughter. At this point additional tests were more demonstrative and tended to fill mnestic gaps with performed. false memories. First, the ability to name visually presented objects In order to check the presence of frontal implications, and actions was tested. the Wisconsin Card Sorting Test [18] was administered: The stimuli used in the action-object picture nam- the score obtained by the patient was comparable to the ing task were taken from the Center for Research in one of the normal population. Language-International Picture Naming Project corpus [CRL-IPNP, 2]. These were 60 actions and 60 objects, with half (30) of the items in each category being “easy” 3. Discussion and half “difficult”. The patient scored perfectly on object naming (60/60 items correctly named), and very We describe a patient who suffered for a sudden well on verb naming (59/60 items correctly named). amnesia of uncertain nature. Even if he appears to be Second, in order to test the ability to learn and retain similar to TGA patients, his clinical features do not items not semantically associated, the list of couple of endorse this aetiology. First, TGA is often associated words taken from Baddeley et al. was administered [1, to evidence of cerebral anomalies (i.e. hypoperfusion 21]. Our patient required six trials to correctly recall the in the right temporal and parietal regions, as in Venneri eight word-pairs, and his performance after the delay (1998) [25]), absent in A.M.; second, by definition, the was excellent too (8 items recalled), suggesting good amnesic syndrome have to be “transient”, namely the learning and retention abilities. patients completely and spontaneously recover after a The patient was discharged in good clinical condi- certain period of time. In our case, instead, the amnesia tions aside from the memory impairment on his per- is unmodified after one year. Third, in TGA cases the sonal history, with Aspirin 300mg qd and diuretics to amnesia is not accompanied by loss of personal identity, regulate his arterial blood pressure. and the entity of autobiographic amnesia is temporally After two weeks the patient was recovered again to graded, with old memories being less affected than undergo a follow-up session. In this circumstance H20 recent memories. In A.M., by opposite, both these two brain PET scans demonstrated hypometabolism in the conditions were unsatisfied. right globus pallidus and putamen. Neuropsychologi- The most intriguing feature of our patient’s history is cal evaluation after two months was substantially un- the selective lasting amnesia for autobiographic events. In fact, not all his past was lost, but only some specific modified, with the exception of recovered construc- topics. On follow-up assessment, he was able to report tional abilities and short-term spatial memory. some events that he had ignored on initial assessment, but he acknowledged to have re-learnt them. 2.4. 9 months follow-up assessment There is a great difference between “remember” and “know”: the first implies the ability to re-experience When he returned home his behaviour was com- the event, the second is a quit “cold” recall, which could pletely unaffective towards his wife and daughter; he result from learning, and it is usually devoid of any demonstrated no interest in his favourite hobbies (i.e. emotional component [3,6,10,23]. his boat) and refused to turn over the pages of his wed- Actually our patient was able to ‘know’, but he still ding photo album, as his wife suggested in order to could not remember. stimulate his recall. One month later, the patient re- For what concerns the selectivity of amnesia, studies turned to work, even if he reported difficulties in re- on patients with different profiles of recollection based membering dates of meetings and specific work-related on memories’ emotional value, investigated the effects items concerning taxes (eg VAT calculations). Since of unilateral damage to the medial temporal lobe [4]. the episode he needs to mark each meeting on a diary A difference based on which side is affected has been 16 C. Repetto et al. / Persistent autobiographical amnesia: A case report found: patients with right temporal lobectomy recall aetiologies as definite categories, the author suggested less unpleasant memories when compared with those an interpretation beside a continuum, where “organic” with left temporal lobectomy. The same results have and “psychogenic” are the two edges, and psychosocial been demonstrated with respect to unpleasant memo- factors interact deeply with brain systems. ries with high intensity of emotional activation, demon- This case report is specially important for our knowl- strating a role of emotional components in autobio- edge on memory organization because of peculiarity graphic memories recollection. Whether the same dis- of his clinical features: the patient shows a clear and tinction can be made in amnesic patients without rec- steady autobiographical memory deficit against the ab- ognizable cerebral lesions, as A.M., is so far unknown. sence of cerebral dysfunction in those sites involved in The normal MRI data led us to consider the amnes- memory processing. tic syndrome as a voluntary behaviour of our patient Therefore, this is a clear example that confirm and (malingering). However, there wasn’t any direct intel- justify the need of a third nosographic entity between ligible reward the patient could obtain from simulation: organic and psychogenic amnesia [8]: in this perspec- he had no legal incentives, economic difficulties and tive, the term “functional”, as formulated before, is a psychosocial conflicts. By contrast, both his private good candidate. and working life have been negatively affected by the event. A psychogenic origin for the disorder was also con- Acknowledgement sidered but our patient shares only one point with those We wish to thank the patient and his wife. necessary for diagnosis of psychogenic amnesia, that is the sudden fugue from his environment. The remaining criteria are however apparently not fulfilled [11,14]: References there is no past history of psychiatric disorders, there are no stressful or conflictual situations declared nor an [1] A. Baddeley, C. Papagno and G. Vallar, When Long-Term highly emotional event known. Despite these observa- Learning Depends on Short-Term Storage, Journal of Memory tions, we must take into consideration the possibility and Language (1988), 586–595. [2] E. Bates, E. Andonova, S. D’Amico, T. Jacobsen, K. Kohnert, that such informations could be not carefully collected C.C. Lu, A. Szkely, N. Wicha, K. Federmeier, D. Herron, G. or investigated during the interview with relatives, as Iyer, T. Pechmann, A. Devescovi, T. Mehotcheva and C. Pleh, indicated by Kopelman (2000) [12]. Introducing the CRL International Picture-Naming Project Without excluding the former hypothesis, but pre- (CRL-IPNP). Center for Research in Language Newsletter, La Jolla: University of California San Diego. 1 (2000). suming the accuracy of the psycho-social investigation, [3] P.J. Bayley, J.J. Gold, R.O. Hopkins and L.R. Squire, The the most fitting diagnosis seems to be the “functional neuroanatomy of remote memory, Neuron 5 (2005), 799–810. retrograde amnesia”. [4] T.W.Buchanan, D. Tranel and R. Adolphs, Memories for emo- In the past, functional amnesia indicated the cases of tional autobiographical events following unilateral damage to medial temporal lobe, Brain Pt 1 (2006), 115–127. focal retrograde amnesia (FRA), in which psychogenic [5] E. Capitani, S. Della Sala, R.H. Logie and H. Spinnler, Re- origin was suspected or verified, so that this label rep- cency, primacy, and memory: reappraising and standardising resented the opposite of organic amnesia. In particular, the serial position curve, Cortex 3 (1992), 315–342. [6] G. Dalla Barba, M.C. Mantovan, E. Ferruzza and G. Denes, functional amnesia was invoked when there wasn’t any Remembering and knowing the past: a case study of isolated detectable brain lesion supporting the clinical memory retrograde amnesia, Cortex 1 (1997), 143–154. deficit. [7] E. De Renzi, What does psychogen mean?, Cortex 4 (2002), Recently this distinction has been criticised [7,8,20], 678–681. [8] E. De Renzi, F. Lucchelli, S. Muggia and H. Spinnler, Is and many authors refer to the term “functional” to in- memory loss without anatomical damage tantamount to a psy- dicate the block of a function. From this point of view chogenic deficit? The case of pure retrograde amnesia, Neu- the distinction is between lesional and non lesional ae- ropsychologia 6 (1997), 781–794. tiology of amnesia, with functional amnesia being ap- [9] M.F. Folstein, S.E. Folstein and P.R. McHugh, “Mini-mental state”. A practical method for grading the cognitive state of plicable when there is no evidence of brain injuries, patients for the clinician, J Psychiatr Res 3 (1975), 189–198. but a block of a function is suspected, triggered both [10] M. Hirano, K. Noguchi, T. Hosokawa and T. Takayama, I by physical or psychic trauma [24]. Kopelman [12, cannot remember, but I know my past events: remembering 13] in fact, proposed a composite model of autobio- and knowing in a patient with amnesic syndrome, J Clin Exp Neuropsychol 4 (2002), 548–555. graphic amnesia that takes into account both organic [11] M.D. Kopelman, Amnesia: organic and psychogenic, Br J and psychogenic mechanisms: instead considering the Psychiatry (1987), 428–442. C. Repetto et al. / Persistent autobiographical amnesia: A case report 17

[12] M.D. Kopelman, Focal retrograde amnesia and the attribu- [19] M.D. Lezak, D.H. Howieson and D.W. Loring, Neuropsycho- tion of causality: an exceptionally critical review, Cognitive logical Assessment, University Press,Oxford, 2004. Neuropsychology 7 (2000), 585–621. [20] H.J. Markowitsch, Functional retrograde amnesia – mnestic [13] M.D. Kopelman, Disorders of memory, Brain Pt 10 (2002), block syndrome, Cortex 4 (2002), 651–654. 2152–2190. [21] C. Papagno, T. Valentine and A. Baddeley, Phonological short- [14] M.D. Kopelman, H. Christensen, A. Puffett and N. Stanhope, term memory and foreign-language vocabulary learning, Jour- The great escape: a neuropsychological study of psychogenic nal of Memory and Language (1991), 331–347. amnesia, Neuropsychologia 6 (1994), 675–691. [22] D.L. Schacter, P.L.Wang, E. Tulving and M. Freedman, Func- [15] M.D. Kopelman, B.A. Wilson and A.D. Baddeley, The autobi- tional retrograde amnesia: a quantitative case study, Neuropsy- ographical memory interview: a new assessment of autobio- chologia 5 (1982), 523–532. graphical and personal semantic memory in amnesic patients, [23] S. Steinvorth, B. Levine and S. Corkin, Medial temporal lobe J Clin Exp Neuropsychol 5 (1989), 724–744. structures are needed to re-experience remote autobiographi- [16] M. Kritchevsky, J. Chang and L.R. Squire, Functional amne- cal memories: evidence from H.M. and W.R, Neuropsycholo- sia: clinical description and neuropsychological proﬁle of 10 gia 4 (2005), 479–496. cases, Learn Mem 2 (2004), 213–226. [24] A. Stracciari, K. Mattarozzi, C. Fonti and M. Guarino, Func- [17] M. Kritchevsky, J. Zouzounis and L.R. Squire, Transient tional focal retrograde amnesia: lost access to abstract autobi- global amnesia and functional retrograde amnesia: contrast- ographical knowledge?, Memory 7 (2005), 690–699. ing examples of episodic memory loss, Philos Trans R Soc [25] A. Venneri and P. Caffarra, Transient autobiographic amnesia: Lond B Biol Sci 1362 (1997), 1747–1754. EEG and single-photon emission CT evidence of an organic [18] M. Laiacona, M.G. Inzaghi, A. De Tanti and E. Capitani, etiology, Neurology 1 (1998), 186–191. Wisconsin card sorting test: a new global score, with Italian [26] D. Wechsler, Wechsler Adult Intelligent Scale-III, The Psy- norms, and its relationship with the Weigl sorting test, Neurol chological Corporation, San Antonio, 1997. Sci 5 (2000), 279–291.